Study Questions:

What are the outcomes of complete neonatal repair as compared with staged procedure for neonates with tetralogy of Fallot (TOF)?

Methods:

A retrospective cohort study was performed using the Pediatric Health Information System (PHIS) database. The study population consisted of PHIS patients with TOF who underwent an initial procedure in the first 30 days of life between January 1, 2004 and March 31, 2015. The primary outcome was death during 2-year follow-up after the initial procedure. Inverse probability-weighted Cox and logistic regression models were used to examine the association between surgical approach group and mortality for accounting for patient- and hospital-based factors.

Results:

A total of 2,363 patients were studied, of which 1,032 underwent complete repairs and 1,331 underwent staged repairs. Complete neonatal repair was associated with a significantly higher risk of mortality during the 2-year follow-up period (hazard ratio, 1.51; 95% confidence interval [CI], 1.05-2.06) and the initial hospital admission (odds ratio, 1.72; 95% CI, 1.15-2.62). Postoperative cardiac complications were more common in the complete repair group and accounted for the differences in 30-day and 2-year mortality.

Conclusions:

The authors concluded that complete surgical repair for neonates with TOF is associated with a significantly higher risk for early and 2-year mortality as compared with the staged approach, after accounting for patient and hospital characteristics.

Perspective:

This important study looked at the differences in outcome between complete neonatal repair and initial palliation with staged repair for neonates requiring intervention for TOF. Potential benefits of early complete repair include avoidance of shunt physiology and associated cyanosis as well as avoiding a second intervention. On the other hand, early complete repair can be associated with significant postoperative morbidity including arrhythmias and severe volume overload. This study showed lower early and 2-year mortality for patients undergoing staged repair. A significant limitation of this study is the possibility of not accounting for all potential confounders. The study comprehensively assessed for both patient- and surgical center-level factors. Additionally, E-value calculations indicated that only the presence of very strong unmeasured confounders could explain the association seen in the study.

A final point to note is the relatively high mortality seen for patients followed in the study. We generally think of TOF as associated with low early surgical mortality and excellent long-term outcomes. In this study, 12.5% of patients in the complete repair group and 10.8% of patients in the staged group had died by 2 years of age. Although this may in part be accounted for by noncardiac factors such as prematurity, low birthweight, or noncardiac syndromes, the study suggests that patients requiring intervention for tetralogy of Fallot in the neonatal period remain a high-risk group.